15例成人Ph染色体和/或BCR-ABL阳性混合表型急性白血病的临床及实验室特征分析

来源 :中国实验血液学杂志 | 被引量 : 0次 | 上传用户:a137167443
下载到本地 , 更方便阅读
声明 : 本文档内容版权归属内容提供方 , 如果您对本文有版权争议 , 可与客服联系进行内容授权或下架
论文部分内容阅读
本研究总结成人Ph染色体和/或BCR-ABL融合基因阳性的混合表型急性白血病(Ph+MPAL)患者的临床和实验室特征。回顾性分析2009年1月-2012年9月在我院诊治的15例Ph+MPAL的特征及细胞形态学、免疫学、细胞遗传学和分子生物学检测以及临床随访结果。Ph+MPAL的诊断采用WHO-2008分类标准。结果表明,Ph+在同时期总MPAL群体中的检出率为17.2%(15/87)。15例患者中男性7例、女性8例;中位年龄为51(16-81)岁;初诊时中位WBC计数为69(12.7-921)×109/L。形态学检查显示为急性髓系白血病(AML)者2例(13.3%),急性淋巴细胞白血病(ALL)者6例(40.0%)、杂合型白血病(HAL)者7例(46.7%)。免疫学分析表明,此15例患者均为B淋系和髓系混合表达,93.3%患者的白血病细胞表面有CD34的表达,其中64.3%(9/14)的患者CD34表达率在60%以上。染色体R显带技术检测显示,正常核型者2例(13.3%),单纯Ph+者8例(53.3%),Ph+伴附加异常者5例(33.3%)。附加的染色体异常分别为-7(2例)、+Ph(1例)、dic(7;9)(1例)、i(8q)和-16(1例);有残余正常分裂相者4例。多重巢式PCR检测发现,e1a2型融合基因6例(40.0%)、b2a2或b3a2型共9例(60.0%)。7例Ph+MPAL患者中有4例检测到IKZF1基因缺失。本组中10例患者诱导治疗一疗程后完全缓解(CR)率为70.0%,其中联合酪氨酸激酶抑制剂(TKI)组的CR率(83.3%;5/6),优于单纯化疗组(50.0%;2/4),但差异无统计学意义(P>0.05)。随访至今,6例患者由于疾病复发或进展而死亡(60.0%;6/10)。异基因造血干细胞移植(allo-HSCT)组患者总生存优于单纯化疗组(P=0.004)。结论:Ph+MPAL主要为B淋系和髓系混合表达,多数年龄较大,高表达CD34抗原。在BCR-ABL融合基因亚型和基因突变方面类似于Ph+急性白血病。初诊时WBC计数是独立的预后因素,复发进展仍是此类患者死亡的主要原因。联合TKI和allo-HSCT或许能改善预后,有必要进行前瞻性、多中心的临床试验来验证。 This study summarizes the clinical and laboratory characteristics of patients with mixed-phenotype acute leukemia (Ph + MPAL) who have positive Ph chromosome and / or BCR-ABL fusion gene. The characteristics of Ph + MPAL in 15 cases diagnosed and treated in our hospital from January 2009 to September 2012 were analyzed retrospectively. The results of cell morphology, immunology, cytogenetics and molecular biology and clinical follow-up were also analyzed. Ph + MPAL diagnosis using WHO-2008 classification criteria. The results showed that the detection rate of Ph + in the total MPAL population of the same period was 17.2% (15/87). Seven of 15 patients were male and 8 were female; the median age was 51 (16-81) years; median WBC count was 69 (12.7-921) x 109 / L at first visit. Morphological examination revealed 2 cases (13.3%) of acute myeloid leukemia (AML), 6 cases (40.0%) of acute lymphoblastic leukemia (ALL) and 7 cases (46.7%) of heterozygous leukemia (HAL). Immunological analysis showed that all 15 cases were mixed with B lymphoid cells and myeloid cells. The expression of CD34 on the surface of leukemia cells in 93.3% of the patients, of which 64.3% (9/14) were over 60%. Chromosome R banding technique showed that there were 2 cases (13.3%) with normal karyotype, 8 cases (53.3%) with Ph + alone, and 5 cases (33.3%) with Ph + abnormalities. Additional chromosomal abnormalities were -7 (2), + Ph (1), dic (7; 9) (1), i (8q) and -16 example. Multiple nested PCR showed that e1a2 fusion gene was found in 6 cases (40.0%) and b2a2 or b3a2 in 9 cases (60.0%). IKZF1 gene deletion was detected in 4 of 7 Ph + MPAL patients. In this group, the complete remission (CR) rate was 70.0% in 10 patients after one course of induction therapy. The CR rate (83.3%; 5/6) in combination with tyrosine kinase inhibitor (TKI) group was better than that in chemotherapy alone group (50.0%; 2/4), but the difference was not statistically significant (P> 0.05). Up to now, 6 patients died of recurrence or progression of the disease (60.0%; 6/10). The overall survival of allogeneic hematopoietic stem cell transplantation (allo-HSCT) group was better than that of chemotherapy alone group (P = 0.004). Conclusion: Ph + MPAL is predominantly B lymphoid and myeloid mixed expression, most of which are older and have high expression of CD34 antigen. Similar to Ph + acute leukemia in BCR-ABL fusion gene subtypes and gene mutations. WBC count at initial diagnosis is an independent prognostic factor and the progression of relapse is still the leading cause of death in such patients. Combined with TKI and allo-HSCT may improve the prognosis, it is necessary to carry out prospective, multicenter clinical trials to verify.
其他文献
本文从硬件和软件两方面详细阐述了以LabVIEW为主要工具开发的一套用于检测非线性射频信号的虚拟仪器的原理及其主要结构;文章讨论了非线性射频信号的特点,阐述了硬件选择的
本文论述了弯杆头部专用无心磨床从设计到生产加工中存在的各方面的问题及为解决这些问题而进行的改造,如砂轮修正器、数控系统、导轮修正器等的改造等.
针对密齿端面铣刀夹紧空间小的特点,设计制造了半压块夹紧新型机构.本文不但分析了半压块夹紧机构的组成和夹紧原理,而且还进行了夹紧力理论分析及应用计算.实践证明,该机构
目的加强医院感染管理,预防控制医院感染的发生。方法建立健全医院感染监控体系,强化医护人员医院感染意识,做好医院感染的各项监测。结果通过规范化管理,降低了医院感染率。结论
随着我国医疗卫生事业的快速发展,绩效管理在医院管理中所发挥的作用也越来越引起关注、重视。有效的绩效管理可以调动全体员工的主观能动性和创造性,不断提高医院的综合竞争力
本文介绍了一种色彩调整技术在彩色打印中的实现.利用三次B-样条曲线拟合技术实现图像色度与亮度的调整.基于多线程的编程技术,实现了打印过程中图像色度与亮度的在线调整,提
会议
鸡西矿业集团公司张辰煤矿西三采区3
期刊
目的研究微格教学法中融入反思学习的模式在基础护理技能教学中的效果。方法以我院2011、2012级286名护生为研究对象,2011级护生为对照组,2012级护生为试验组,对照组采用传统技
护理质量直接关系到患者的生命与健康。笔者所在科室通过建立高素质护理人才培养机制、更新护理质量管理理念、转变护理服务模式、实现制度化、规范化管理,保证了护理工作的安
本文阐述了快速原型制造技术的基本原理,介绍了快速原型制造技术的分类和快速成型工艺,指出了快速成型技术应用和发展趋势.